Hello Everyone,
I really need some help. I have a daughter who’s blood work came back with some interesting findings, and I am not sure what to make of it. Her Hematocrit was high, as was her PT and INR. Her PTT was low. She tested high for Von Willebrand Factor Activity (GP1b) and Factor Vlll Activity, and low for Factor Vll Activity.
The summary says: Von Willebrand Factor Activity was normal with Von Willebrand Factor Antigen level. Prolonged PT/INR with a decreased coagulant Factor Vll level.
If someone tests high for some of it, and low on the other mentioned above, does that mean someone does have it?
Thank you.
Christina,
There are different types of VonWillebrands. Also, there are other types of platelet defects, where some other mechanism is not functioning properly in the clotting process. My Factor VIII always comes up normal and I do respond well to DDAVP but they have not identified exactly which part of the clotting process is defective in me.
My hematologist just told me yesterday that there is some new testing that she can do on me. I may have that done. The other option is to speak to someone at Children's Hospital's Hemophelia Center. I know case worker at the Center wanted to send me to a hemotologist who specializes in these defects. I haven't done that yet because it involves travelling.
What does your daughter's hematologist say about the test results?
Ally
Hello Ally,
Thank you for your reply. We are in the middle of retesting. I think they are looking at part of the clotting process that is not working properly. They ordered a RET, Coagulation, and are doing a Hemostasis Study Smear. They are also doing a VWD study with Closure Time.
Anyone have these tests done, and know what they could mean?
I would also be interested to know if anyone who has it, has bags under their eyes all the time. Not tiredness or allergy. It never seems to go away.
Dear Christina, did you get any new results from the retesting? A long PT test with a normal APTT test sounds like a defect in Factor VII in the Extrinsic Coagulation Cascade and that has several possible blood factor deficiencies. Hemophilia B is total lack of Factor VII. There are also some other platelet abnormalities in that part of the coagulation cascade and some need special tests. I have heard of Factor XIII and Factor V problems but I sure there are others. I suffer from Factor VIII inhibitors and extremely low VWF so I come out vWD type III and/or Hemophilia A.
Keep in touch,
John the elder just out of the hospital after IVIG treatment for inhibitors.
Christina said:
Hello Ally,
Thank you for your reply. We are in the middle of retesting. I think they are looking at part of the clotting process that is not working properly. They ordered a RET, Coagulation, and are doing a Hemostasis Study Smear. They are also doing a VWD study with Closure Time.
Anyone have these tests done, and know what they could mean?
I would also be interested to know if anyone who has it, has bags under their eyes all the time. Not tiredness or allergy. It never seems to go away.
Dear Christina,
I mispoke about Hemophilia B, That version is missing Factor IX.
The Hemostasis study smear is a blood sample thinly smeared across a slide and a human or a robot (Coulter Counter) looks at the number, size and shape of the platelets. The results, compared to Standard Blood (a committee designed that, of course!!) give an indication of qualitative or quantitative platelet abnormalities. Depending on where the abnormalities show up, the diagnosis will be different. Type two vWD typically shows too big, too small or wrong shape VWF platelets, Type one and three vWD show numerical anomalies, hence "quantitative issues". Other platelet disorders will also show in this type of testing.
An interesting reference site is found at: www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/hematology-oncology/disorders-platelets.
The Cleveland site has professional papers so expect to look up a lot of jargon like "Thrombocytopenia" , ischemic, and "Hematolysis", but there are only about ten common jargon bits the docs use to confuse us lay people.
I will try and make a glossary of these and post it.
vWD study test, usually includes VWF antigen, Ristocetin co-factor test, Factor VIII activity test and APTT (a measure of Factor VIII/VWF effectiveness in clotting) . There is a new test called PFA 100 which is not in common use but apparently is more sensitive. The vWD panel identifies vWD as a diagnosis versus other platelet disorders and the smear test helps identify which sub-group of vWD you are dealing with.
Good luck! and tell us how the tests work out.
John the elder
Would someone please teach me how to paste in a URL so you can just click on it!!!